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Congenital and inherited hyperpigmentation disorders

Neelam Vashi, MD
Roopal Kundu, MD
Section Editor
Erik Stratman, MD
Deputy Editor
Rosamaria Corona, MD, DSc


Hyperpigmentation is the darkening or increase in the natural color of the skin, usually due to an increased deposition of melanin (hypermelanosis) in the epidermis and/or dermis. Less frequently, it may be caused by the deposition in the dermis of endogenous or exogenous pigments, such as hemosiderin, iron, or heavy metals.

Hyperpigmentation is a feature of a multitude of clinical conditions, ranging from normal variations of skin color to acquired and inherited syndromes, and is one of the most common reasons for dermatologic consultation, particularly in patients with darker skin types [1-3]. Although hyperpigmentation is not harmful, it can cause significant cosmetic disfigurement and become a persistent psychosocial burden for the patient, due to the limited efficacy of the available treatments.

This topic will review the approach to the patient with congenital and inherited hyperpigmentation disorders. Incontinentia pigmenti and pigmentary mosaicism are discussed separately. Acquired disorders of pigmentation are also discussed separately.

(See "Incontinentia pigmenti".)

(See "Pigmentary mosaicism (hypomelanosis of Ito)".)


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Literature review current through: May 2017. | This topic last updated: Jun 12, 2017.
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